Pediatric emergency medicine trisk 2474 2474
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Infants with elevated direct bilirubin have conjugated hyperbilirubinemia,
which is always pathologic. Elevated direct bilirubin indicates obstructed
excretion, or cholestasis, usually due to biliary obstruction, hepatocellular
pathology, or metabolic disorder. Hepatitis, and in particular, idiopathic neonatal
hepatitis, is a common cause of conjugated hyperbilirubinemia. This must be
distinguished from infectious hepatitis, including hepatitis B, rubella,
cytomegalovirus, toxoplasmosis, coxsackie virus, echovirus, HSV, syphilis,
Listeria, and tuberculosis. In addition, systemic infectious disease can also result
in a toxic hepatitis, particularly systemic infections due to Escherichia coli,
Pneumococcus, Proteus, and Salmonella. Prolonged parenteral alimentation is
another common cause of conjugated hyperbilirubinemia that can persist several
months beyond the cessation of parenteral nutrition. Metabolic disorders of the
liver that can also result in conjugated hyperbilirubinemia include alpha 1antitrypsin deficiency, galactosemia, tyrosinemia, fructosemia, glycogen storage
diseases, lipid storage diseases, cerebrohepatorenal syndrome, trisomy 18, cystic
fibrosis, hemochromatosis, and idiopathic hypopituitarism.
Obtain CBC, blood culture, albumin, LFT’s PT/PTT, hepatitis serologies,
urinalysis, urine culture, and urine-reducing substances. Biliary atresia and
neonatal hepatitis are the most common etiologies and patients with biliary atresia
have better outcomes the earlier they are diagnosed. Neonates presenting with
elevated direct bilirubin with microcephaly, seizures, organomegaly, or petechiae
should be evaluated for congenital TORCH (toxoplasmosis, other infections,
rubella, CMV, herpes simplex) infections.
Management. Determine if the infant has indirect or direct hyperbilirubinemia.
Infants with direct (conjugated) hyperbilirubinemia or acholic stools require
admission for further diagnostic evaluation. Treatment of neonatal hepatitis is
supportive, with supplementation of the fat-soluble vitamins. Prevention of
hepatitis B infection requires early administration of HBIG and HBV vaccines to
prevent chronic infection in exposed newborns. Septic infants should be treated
with broad-spectrum antibiotics, typically with rapid normalization of
hyperbilirubinemia. While some of the metabolic disorders (galactosemia,
tyrosinemia, fructosemia, and the storage diseases) may be palliated with dietary
restrictions, most progress to chronic cirrhosis and liver failure.
Indirect Hyperbilirubinemia. Infants with physiologic jaundice can be
discharged if they do not meet criteria for phototherapy or exchange transfusion.
The Subcommittee on Hyperbilirubinemia of the AAP publishes guidelines for
management of hyperbilirubinemia in healthy term infants according to total
